Juvenile Idiopathic Arthritis

Author(s): Glendaliz Bosques, MD and Mani P. Singh, MD

Originally published: November 10, 2011 Last updated: July 28, 2020

DISEASE/DISORDER:

Definition

Juvenile idiopathic arthritis (JIA), previously known as juvenile rheumatoid arthritis (JRA) or juvenile chronic arthritis (JCA) is a form of arthritis (i.e., swelling or limitation in range of movement of a joint with warmth, pain or tenderness) of unknown etiology that occurs for at least 6 weeks in children younger than 16 years of age.¹ JIA is a diagnosis of exclusion.² It is thought to be a multifactorial autoimmune disease associated with certain environmental and genetic factors. It can continue into adulthood, causing significant disability, poor functional outcomes, and decreased quality of life.³

Etiology

JIA appears to be influenced by both genetic and environmental factors. Our current evidence stems from family and twin studies. The (human leukocyte antigen) HLA-A2 allele and HLA-B27 allele have been implicated in certain forms of JIA, especially in subtypes with earlier onset. Recent genomic studies in Europe have identified new genetic loci in non-HLA regions.⁴ There may be involvement of the TNF-alpha, PTPN22, MIF, SLC11A6, and WISP3 genes as well, but the current evidence is inconclusive.⁵

In addition to HLA associations, both cell-mediated and humoral-immunity play a role. Multiple genes relating to inflammation and immune regulation are believed to be involved. There is evidence of autoimmune dysregulation, with positive antinuclear antibody (ANA) in 40% of patients. However, evidence from treatments designed to block the inflammatory cascade indicate that systemic inflammation may play a greater role.

In addition to strongly indicating a genetic component, studies with monozygotic twins have elucidated that environmental factors, such as infections, also play a role. Concordance rates are closer to 25-40% (as opposed to 100%) in these twins.⁵ Repeated infections and antibiotics during the first two years of life are associated with JIA in multiple studies.^6,7The most commonly implicated pathogens include Parvovirus B19, Epstein-Barr virus, enteric bacteria, Chlamydia species, Bartonella henselae, Mycoplasma pneumoniae, and Streptococcus pyogenes. External antigens may provoke multiple antigen-specific pathways, including cytotoxic T-cell responses, proinflammatory cytokine production, and the complement cascade, increasing the risk of immune-mediated arthritis during childhood.⁵ However, it is unclear whether this is due to pathogenic alterations of the microbiome or changes to our immunologic cells.

Epidemiology including risk factors and primary prevention

JIA is the most common chronic rheumatic disease in children.⁸ 0.07 to 4.01 per 1000 children are affected worldwide.¹ The average yearly incidence is approximately 2-20 cases per 100,000 with a prevalence of 16-150 cases per 100,000 people.⁸ Depending on the subtype, girls may be more affected than boys (two to four times more).⁹ Differences in age and sex can also be seen within subtypes of JIA. For example, in rheumatoid factor (RF) positive JIA, females may outnumber males 9:1 compared to overall JIA ratios.

Although variable, one study showed that the average age of onset for JIA was 5.5 years old. Improvements in modern diagnostic practices has led to a decrease in the average age of JIA as cases are being diagnosed earlier.¹⁰

Recent epidemiologic surveys have shown distinctions in geography and ethnic groups. Oligoarthritis is predominant in western countries but rare in areas such as India, South Africa, and the Middle East, where the polyarticular form of JIA is more common. Greater incidences of enthesitis-related arthritis are present in India, Taiwan, parts of Mexico, and Canada. Black Americans have a higher rate of RF positive polyarthritis whereas European ancestry may predispose children to higher rates of ANA positive JIA.¹¹

A multinational study, named the Epidemiology, Treatment, and Outcome of Childhood Arthritis (EPOCA Study), is currently ongoing to obtain epidemiologic data on JIA subtypes. Preliminary data shows a younger age of onset in Western Europe and a higher prevalence of uveitis in this population. While there may be lower rate of incidence in Asian populations, there may be higher rates of systemic arthritis in these patients. Thus far, children in Africa and Eastern Europe have been found to have higher levels of disease activity as well as a lower frequency of inactive disease.¹¹ Our understanding of the etiology of JIA continues to evolve to this day.

Patho-anatomy/physiology

JIA is not a single disease but rather a term used to identify a heterogeneous group of illnesses with distinct clinical phenotypes, disease courses, outcomes, and genetic backgrounds. The subtypes of juvenile arthritis can be differentiated from one another based on the number of affected joints, underlying biochemistry, and the presence of associated systemic comorbidities.⁵

JIA is an inflammatory syndrome characterized by chronic synovial inflammation with B-lymphocytes. Macrophage and T-lymphocyte invasion occurs along with cytokine release and further synovial proliferation. There is a major pathogenic role for the overproduction of interleukin-6 and interleukin-1.⁸ Eventually, the thickened synovium (pannus) contributes to joint destruction. Within the synovial fluid itself, interleukin-18 expression has consistently been found to be abnormal.

JIA Subtypes (International League of Associations for Rheumatology [ILAR] classification)^12,13

Oligoarticular
- Persistent oligoarthritis: affecting fewer than four joints during first six months of disease
- Extended oligoarthritis: affecting one to four joints during first six months of disease but greater than five joints after six months of disease
- Onset: early childhood, Females > Males¹⁴
- Most common (50%-60%), best prognosis
- Larger extremity joints, especially knees, most commonly affected
- Iritis, especially in girls
- Polyarticular
  - RF negative polyarthritis: affecting five or more joints during the first six months of disease with negative RF test
    - More frequent in girls and with symmetric small joint involvement and severe inflammation
  - RF positive polyarthritis: affecting five or more joints during first six months of disease with two or more tests for RF positive at an interval of at least three months
    - Variable prognosis, more aggressive disease course
    - May have positive antibodies to cyclic citrullinated peptides (anti-CCP)⁸
  - Occurs in 25%-40% of cases⁵
  - Onset: RF positive in late childhood, RF negative is often younger than six years; Females > Males
  - Symmetric involvement of small joints of hands and feet
  - Cervical spine and temporomandibular joint (TMJ) may be affected
- Systemic
  - Arthritis in one or more joints with or preceded by fever of at least two weeks duration and daily fever (for at least three days), and accompanied by at least one of the following signs: non-fixed erythematous skin rash, generalized lymph node enlargement, hepatomegaly or splenomegaly, serositis
  - About 5-15% of cases
  - Onset: any age, Female = Males
  - Quotidian pattern of high spiking fevers
  - Rash is evanescent, salmon colored, macular, accompanies fever
  - Arthritis occurs later, a month or longer
  - Formerly known as Still’s Disease or systemic juvenile rheumatoid arthritis
- Psoriatic
  - Arthritis and psoriasis with at least two of the following: dactylitis, nail pitting or onycholysis, psoriasis in a first-degree relative
  - 3-10% of patients
  - Onset: biphasic with early peak younger than six years, late peak after six years of age, Female > Male
- Enthesitis-Related
  - Enthesitis at the sites of tendon insertion onto bone with at least two of the following: presence or history of sacroiliac joint tenderness or lumbosacral pain, HLA-B27 antigen positive, onset of arthritis in male over six years of age, acute symptomatic anterior uveitis, history of ankylosing spondylitis, enthesitis-related arthritis, sacroiliitis with inflammatory bowel disease, or acute anterior uveitis (Reiter’s syndrome) in a first-degree relative
  - In 30-40% of patients, disease progresses to affect sacroiliac joint
  - Onset: late childhood, Males > Females
- Undifferentiated
  - Patients do not meet inclusion criteria for any category, or they meet criteria for more than one subtype
  - 10-20% of patients may fall under this category

Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time)

Disease progression can be variable given the spectrum of JIA and is often dynamic as well, particularly in the first six months from onset.⁹ In a recent cohort study, 32.8% of JIA patients were in remission (defined as no disease activity and the absence of medications for at least 6 months) after a mean follow up of 10 years. RF positive JIA appears to have lower levels of remission compared to other subtypes of JIA.

As children grow into adulthood, studies have noted the presence of severe disability in as many as 42.9% of patients.^3,9 Unemployment was also found to be twice as high than the national average, despite educational achievement in these patients. Standardized mortality ratios are higher in both male (3.4) and female (5.1) patients with JIA.⁹

New onset/acute:
- Oligoarticular
  - Peak onset: pre-school age
  - Joint pain, swelling, tenderness (especially in large joints such as the knees)
  - Child may limp without complaint or gait abnormalities more prominent in the morning
- Polyarticular
  - Age of peak onset:
    - RF positive: > 10 years
    - RF negative: 1-3 years
  - Pain, swelling, tenderness in greater than five joints
- Systemic
  - No peak age of onset
  - Clinical symptoms and frequencies: Fever (98%), Arthritis (88%, majority with oligoarthritis or polyarthritis), Rash (81%), Lymphadenopathy (31%)
  - Quotidian fever for about two weeks¹⁵
Subacute: Systemic presents with joint pain in the first six months. The other types may resolve in six months.
Chronic/stable: Oligoarticular JIA that lasts more than six months is characterized as “extended”. Complete remission can be expected in most patients. Remaining patients continue to have symptoms into adulthood, with about 30% having significant disability.
Pre-terminal: Only in very severe cases, affecting cervical spine or causing significant debility.

Specific secondary or associated conditions and complications

Anorexia, weight loss, growth failure
Cardiopulmonary involvement (pericarditis, pleural effusions with rare complication of tamponade, if large enough)
Anemia
Decreased physical activity contributes to weakness, obesity (which increases load on joints)
Atlantoaxial instability associated with cervical spine involvement increases risk of spinal cord injury
Concerns have been raised about increased rates of cancer (does not seem to be related to treatment with biologic agents as was once thought)
Uveitis, specifically iridocyclitis. More common in females affected with oligoarticular arthritis and in patients less than six years of age with a positive ANA. Usually asymptomatic, but if not diagnosed early may result in permanent blindness.
Growth disturbances, including growth retardation and accelerated growth, resulting in conditions such as leg length discrepancies
Micrognathia if TMJ arthritis disturbs the growth plate (particularly in polyarticular JIA)
Suppression of the immune system by disease modifying antirheumatic drugs (DMARDs) increases the risk of infections
Osteopenia and osteoarthritis
Fractures
Hepatomegaly, splenomegaly, lymphadenopathy (particularly in systemic JIA subtype)
Rare pulmonary manifestations may include interstitial lung disease or pulmonary hypertension¹⁶
Macrophage Activation Syndrome (MAS) resulting from uncontrolled activation and proliferation of T-lymphocytes and macrophages is a complication with systemic JIA. It is a life-threatening condition resulting in persistent fever, pancytopenia, hepatosplenomegaly, and coagulopathy.
Pain is an incredibly common symptom of JIA but not one that is explained by disease activity alone. Recent studies have shown that older age of onset, poor function or increased disability, and longer disease duration were all associated with consistently higher pain levels. Patients at risk of chronic pain should be identified early and targeted with multidisciplinary pain management interventions.¹⁷

ESSENTIALS OF ASSESSMENT

History

Joint pain and swelling, often noted incidentally after trauma
Stiffness worse in morning or after naps and lasts more than fifteen minutes
Symptoms better later in the day
Persistence of symptoms for at least six weeks in a six-month period
History of fever without other cause, in absence of joint symptoms; may be three days, with a double spike pattern of high temperatures
Child refusing to walk, or using hands to walk
Pain with ambulation, “gelling” sensation (stiffness after a joint remains in one position for a prolonged period), joint swelling, and difficulty with buttons or writing
Photophobia, pain, redness, headache, and visual changes
Isolated musculoskeletal pain is generally not JIA

Differential diagnoses include: Perthes disease, slipped capital femoral epiphysis (SCFE), malignancy (osteosarcoma for joint pain, leukemia for fever combined with joint pain, lymphoma), ankylosing spondylitis, inflammatory bowel disease, septic arthritis, Kawasaki disease, malaria

Physical examination

Musculoskeletal:
- Painful, swollen joints
- Number of joints
  - 1-4: Oligoarticular
  - 5 or more: Polyarticular
  - Any joint can be affected, including small joints of the fingers, sternocostal, vertebral joints (including cervical), and the jaw. Knees, wrists and ankles more affected in polyarticular.
  - Note if deformity present with chronic disease.
- Range of motion: decreased. Note if contractures.
Extremities: limb length discrepancy. Systemic disease may present with lymphadenopathy.
Gait: antalgic limp
Head, eyes, neck: Check eyes for uveitis in girls with positive ANA and oligoarticular JIA. Receding chin from early ossification of the mandible (micrognathia)
Skin: evaluate for rashes. Document type and location.
Gastrointestinal: systemic disease may have hepatomegaly and/or splenomegaly
Cardiovascular: may have pericarditis, myocarditis (muffled heart sounds, pericardial friction rub)

Functional assessment

Childhood Health Assessment Questionnaire (CHAQ) Disability Scale: measures activities of daily living (ADL) and independence
Juvenile Arthritis Self-Report Index: measures self-care, mobility, school and extracurricular involvement
Juvenile Arthritis Functional Assessment Report (JAFAR): focuses on performance of physical tasks, has parental and self-report versions
Health-Related Quality of Life (HRQoL): used as a measure of functional outcome and to measure responses to treatment. Includes scores for:
- Psychological Functioning (MCS)
- Physical Functioning (PCS)¹⁸
Health Utility Index Mark 3 (HUI3) and the Medical Outcomes Short Form 36 (SF-36) have been utilized to assess functional improvement in patients with JIA¹⁸
Patient-Reported Outcomes Measurement Information System (PROMIS): includes functional measures such as mobility, upper extremity function, fatigue, and pain interference¹⁹

Laboratory studies

No specific laboratory studies definitively diagnose JIA, thus highlighting the importance of a comprehensive history and physical examination. Blood testing for inflammatory markers (Erythrocyte sedimentation rate, C-reactive protein) and autoimmune markers (RF, HLA-B27, ANA) are recommended.¹

RF‑positive in 3%-5% of polyarticular subtype
ANA positive in 80% of oligoarticular subtype, associated with uveitis
Serial trending of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) help gauge effectiveness of treatment
Complete blood count (CBC) to evaluate for anemia of chronic disease and leukocytosis
Joint aspiration to rule-out septic arthritis
Hyperferritinemia may be found in systemic JIA²⁰

Imaging

Plain films, magnetic resonance imaging (MRI), and ultrasound can be used to detect joint changes.

Plain films: periarticular soft tissue swelling, overgrown or ballooned epiphyses, periostitis
- Later, may show erosive disease, joint space loss
MRI: high sensitivity to detect bone damage and damaged cartilage
Ultrasound: useful for assessing number of joints involved and differentiating between tendon and articular involvement
Dual-energy x-ray absorptiometry (DEXA): to identify osteoporosis
- Particularly useful in patients requiring long term corticosteroids or those with decreased levels of physical activity

Supplemental assessment tools

Physician and parent global assessments of overall disease activity
Steinbrocker functional classification rates the extent of physical disability in patients^21,22

Early predictions of outcomes

Poorer prognosis associated with:
- Hip or cervical spine involvement, joint space erosion, positive RF, and positive anti-CCP antibodies
- Early hand involvement and radiographic changes of carpal length are also associated with poorer prognosis
- Systemic and polyarthritis with RF positivity have the highest incidence of advanced joint damage and long-term disability
- Inactivity and poor aerobic and musculoskeletal fitness. Affected children are at an increased risk of cardiovascular disease, diabetes mellitus, depression, hypertension, obesity, osteoarthritis/osteoporosis²³
Positive ANA may be associated with less disability⁹
Early intensive therapy in JIA may improve long term disease outcomes, including prevention of cumulative joint damage

Environmental

More severe cases may require home modifications, including ramps and roll-in showers secondary to mobility limitations
Changing doorknobs to handles may help those with advanced hand and wrist involvement
School accommodations based on Section 504 of the Rehabilitation Act
- Regulations to accommodate decreased ability to get from class to class, take notes, or carry books
- Provision of modified school supplies may be necessary, if small joints of hands and grasp affected
- Recommendations for physical education modifications to assist in maintaining student activity, provide joint protection, and energy conservation techniques

Social role and social support system

Data indicates that adolescents with JIA struggle with physical vulnerability, isolation, and managing uncertainty. Many find resilience through taking control of their disease. Sharing of experiences with other adolescents can help foster acceptance and self-growth.²⁴
Family frustration due to delay of diagnosis in some cases
Pain leads to missed school and participation
Quality of life indices for patients were found to decrease with psychological distress, higher disease activity, or when their caregivers reported financial hardship²⁵

Professional Issues

Physicians must ensure they educate patients on their disease course, as many children are eager to learn about their prognosis and functional limitations. Physiatrists must be strong patient advocates in multiple settings, including school, home, and community environments. Sensitivity to patients’ cultural background, ethnicity, gender, age, religious beliefs, and sexual orientation is paramount to building trust across the spectrum of care. Patient safety issues include minimizing medical errors, addressing medication side effects, and providing a safe environment at home and during therapy.

REHABILITATION MANAGEMENT AND TREATMENTS

Available or current treatment guidelines

2019 American College of Rheumatology Recommendations for the Treatment of Juvenile Idiopathic Arthritis
- 2021 guideline updates to 2019 guidelines anticipated in early 2021¹³
American College of Rheumatology 2011 guidelines for therapeutic agents ^26,27
Exercise and Physical Activity Conference Arthritis Working Group 2002 Guidelines²⁸

At different disease stages

New onset/acute

Includes symptom relief:
- Non-steroidal anti-inflammatory drugs (NSAIDs) for pain and decrease of inflammation (typically see improvement in first 1-3 days):
  - Diclofenac
  - Naproxen
  - Ibuprofen
  - Indomethacin
  - Meloxicam
  - Tolmetin²⁹
- Intra-articular steroid injections with triamcinolone hexacetonide is preferred for children
- For uveitis, topical corticosteroids followed by systemic corticosteroids or biologics in resistant cases (such as Infliximab)⁸
Includes rehabilitation strategies that stabilize or optimize function and prepare for further interventions at later disease stages:
- Gentle range of motion
- Cold packs applied to joints
- Supportive orthoses, especially knees and wrists to maintain alignment
- Shoe modifications to support ankles and feet
- Appropriate rest from activities during flares

Subacute

Includes secondary prevention and disease management strategies:
- Prednisone for acute exacerbations or in chronic, resistant cases
- DMARDs such as methotrexate (one of the most studied drugs in JIA, proven to be efficacious), leflunomide, sulfasalazine combined with an NSAID²⁹
- Anti–interleukin (IL)-1 agents include anakinra, canakinumab, rilonacept
- Anti–IL-6 agent tocilizumab
- TNF-α inhibitors like etanercept, infliximab, and adalimumab help reduce flare-ups
  - Repeated annual screening of TB in low risk patients with a negative initial TB screen is not recommended
- T-cell immunologic medications such as abatacept⁸
- CD-20 antigen suppression agent Rituximab²⁹
- Anti-tumor necrosis factor drugs in addition to those above
  - May have severe adverse effects, requiring dose adjustments
Includes rehabilitation strategies to optimize function:
- Range of motion
- Heat, including paraffin and beads for hands
- Hydrotherapy
- Ultrasound modality generally avoided in children due to controversy over growth-plates
- Mobilization to prevent debility
- Joint protection techniques
- Adaptive equipment for mobility (walker with forearm supports if grip is too difficult) and ADLs (adapted silverware, pencil or crayons)
- Adapted computer access

Chronic/stable

Includes secondary prevention and disease management strategies
- Monitor cervical spine
- Supportive orthotics, especially for knees, feet, ankles and wrists
- Jaw guards for sports if jaw involved
Includes palliative strategies
- Supportive nutrition
Includes symptom relief
- NSAIDs
- Complementary and alternative medicine techniques common
Includes rehabilitation strategies to optimize function
- Massage
- Stretching and flexibility
- Yoga
- Exercise program including aquatics
  - Aerobics, while protecting joints
  - Strengthening, isometrics may be better for affected joints
- Participation in sports is not contraindicated
- Joint protection strategies
- Environmental adaptations
- Nighttime resting splints

Patients who present with chronic, persistent and debilitating pain are at high risk for developing Chronic Amplified Pain Syndrome. These patients should be identified early and provided with multidisciplinary pain management to avoid and manage disability, deconditioning, decrease exposure to opioids, improve function, and optimize community participation and educational achievement.³⁰

Pre-terminal or end of life care:

Severe complications, such as cervical spine involvement can lead to severe disability and early death.
Includes symptom relief
- Continued pain relief and supportive care

Role of Corticosteroids

The role of corticosteroids is now more limited as methotrexate and other biological agents have become more accessible and better studied. However, there are many instances when corticosteroids are indicated.

Intraarticular Injections: triamcinolone hexacetonide can treat contractures secondary to synovitis to prevent limb length discrepancies or anatomic deformities. Ultrasound guidance can maximize efficacy and prevent subcutaneous atrophy (caused when steroid preparation is delivered out of the joint space). A recent review found that knee joints may benefit from rest, but wrist joints may not benefit from rest following steroid injections.³¹
Chronic Iridocyclitis: screen all children periodically for iridocyclitis with slit-lamp examinations as it is asymptomatic at onset and early diagnosis can improve outcomes. Periocular steroid injections can be used if there is no response to topical therapy (first line). Systemic steroids are indicated in localized treatment failure.¹⁴
Corticosteroids as a bridge: short courses of corticosteroids can be used to alleviate pain and stiffness in patients waiting for full therapeutic effects of recently initiated therapies or symptoms refractory to other therapies.¹⁴
Systemic JIA: one of the main indications for systemic corticosteroids; if symptoms recur after steroid tapering, methotrexate or biologic agents are typically used (anti-IL-1 or anti-IL-6 inhibitors).¹⁴
Macrophage Activation Syndrome (MAS): MAS is a life-threatening syndrome that occurs in 6-8% of patients and is a form of reactive hemophagocytic lymphohistiocytosis. MAS is a medical emergency and should be promptly recognized and treated.¹⁴

Role of Physical Therapy and Exercise

Exercise therapy is well tolerated and beneficial across various outcome measures in patients with JIA. It is considered an integral component of the standard of care for JIA, as children commonly display balance abnormalities, muscle weakness and atrophy, decreased physical fitness, and may be more sedentary regardless of disease activity.³² Growing evidence shows that exercise does not lead to worsening of JIA symptoms. In fact, the evidence shows that a physical therapy led exercise program can have beneficial effects on multiple outcome measures, including the Child Health Assessment Questionnaire, Pediatric Quality of Live Inventory, pain scores, and muscle strength.

In addition, The Ottawa Panel for Evidence-Based Clinical Practice Guidelines for Structured Physical Activity (2017) recommended Pilates, cardio-karate, home and aquatic exercises in order to improve quality of life, pain, functional ability, range of motion, and decrease the number of active joints affected.³³

Coordination of care

A multidisciplinary team is needed to properly care for patients with JIA. Medical teams commonly include a pediatric rheumatologist, physiatrist, orthopedic surgeon for deformities (i.e., avascular necrosis of the femoral neck), and ophthalmologists. Physical and occupational therapists can address joint range of motion (ROM), pain, strength, and endurance. Occupational therapists may also assist with school and environmental modifications. Vocational therapists can address education and employment plans after high school and college. Psychologists and social workers may assist with coping strategies, self-esteem, and transition into adulthood.

Patient & Family Education

Joint protection
Energy conservation techniques
Need for regular range of motion
Need for maintaining overall health, including nutrition and fitness
Adequately explain pain regimen and any potential adverse effects
Educate on activity restrictions and therapy programs
Patients and families should be aware of complications of the disease or treatments
Atlantoaxial involvement precludes participation in contact sports, as well as gymnastics and jumping on trampolines, due to risk of subluxation/dislocation causing spinal cord injury
Weight bearing status
Recommended medical, surgical, and rehabilitative treatments
Duration of treatment

Emerging/unique Interventions

IMPAIRMENT-BASED MEASUREMENT

Childhood Health Assessment Questionnaire (Discomfort scale)
JADAS (Juvenile Arthritis Disease Activity Score): includes physician’s global assessment of disease activity, parent’s assessment of well-being, ESR, and number of joints with active disease. There are also variations of the JADAS score that can be used:
- JADAS-CRP: replaces ESR with CRP measurement
- Clinical JADAS (cJADAS): does not include ESR or CRP³⁴
Juvenile Spondylarthritis Disease Activity Index (JSpADA): used to measure disease activity by asking patients to rank certain symptoms (arthritis, enthesitis, pain, acute phase reactants, morning stiffness, clinical sacroiliitis, uveitis, back mobility), with higher scores indicating greater disease activity³⁴
Juvenile Arthritis Damage Index (JADI): quantifies the amount of damage, defined as persistent anatomical changes or functional changes, in order to measure morbidity³⁴

MEASUREMENT OF PATIENT OUTCOMES

Juvenile Arthritis Quality of Life Questionnaire: physical function and emotional well being
Juvenile Arthritis Multidimensional Assessment Report (JAMAR) – being used in the EPOCA study¹¹
Juvenile Arthritis Functional Assessment Scale (JAFAS): activity performance and participation³²
Childhood Arthritis Health Profile: physical and psychosocial functioning, and family impact of the disease
Quality of My Life Questionnaire: focuses on separating problems related to JIA versus other issues
Childhood Health Questionnaire: general health, pain, physical activity, self-esteem and family issues
Pediatric Quality of Life Inventory Scales: physical, emotional, social, school function
Patient-Reported Outcomes Measurement Information System (PROMIS): developed by the National Institutes of Health (NIH) for use across a range of conditions to assess self-reported health and has recently been researched in pediatric JIA¹⁹

Translation into practice: Practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills

Leg length discrepancy related to joint damage due to chronic synovitis of involved lower extremity joint may require orthotic adjustments, including shoe lifts
Symptom management with intra-articular steroids can decrease inflammation and help prevent synovial overgrowth
Exercise should be promoted, even in times of disease flare. Regular low to high intensity exercise, including aquatic therapy, has been shown to decrease pain, improve clinical symptoms, and improve quality of life.
Patients with JIA are at high risk for chronic pain and early exposure to opioids. Older age of onset, increased disability or decrease function, and longer disease duration have all been associated with higher pain levels. Patients at risk of chronic pain should be identified early and targeted with multidisciplinary pain management interventions in order to improve long term pain outcomes and decrease opioid dependence.¹⁷

CUTTING EDGE/EMERGING AND UNIQUE CONCEPTS AND PRACTICE

Cutting edge concepts and practice

The EPOCA Study is a multinational study coordinated by the Pediatric Rheumatology International Trials Organization (PRINTO). This study is collecting information from over 9000 patients from over 40 countries with the aim to obtain information on frequency and characteristics of JIA subtypes across the globe. Preliminary data is available while the study is currently ongoing.^4,11
Delayed Gadolinium-enhanced MRI cartilage imaging may be able to assess proteoglycan content within joints before morphological damage is apparent, leading to earlier recognition and treatment of JIA
Smart devices and technologies, including phone applications, are being explored to monitor symptoms and disease progression, to increase adherence to prescribed medications, and to encourage physical activity. These technologies have been implemented in other diseases previously, such as asthma and adult rheumatoid arthritis.³⁵

GAPS IN THE EVIDENCE-BASED KNOWLEDGE

Gaps in the evidence-based knowledge

DMARDs have revolutionized the treatment of JIA, improving the quality of life for patients with joint-related disability. Since these drugs are relatively new to the market, the long-term consequences remain unknown. Since many children with JIA continue to have disease as adults, patients may remain on these agents for decades, necessitating further investigation into their long-term effects.
Adults with JIA often have significant disability. There is a need for high quality transition of care from the pediatric to the adult population, as well as a need for studies researching this transitional population.³

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Original Version of the Topic:

Edward Hurvitz, MD. Juvenile Idiopathic Arthritis. Publication Date: 2011/11/10.

Previous Revision(s) of the Topic

Todd R. Lefkowitz, MD , Sean Bemanian, MD Juvenile Idiopathic Arthritis. Publication Date: 4/21/2016.

Author Disclosure

Glendaliz Bosques, MD
Nothing to Disclose

Mani P. Singh, MD
Nothing to Disclose

Pediatric Rehabilitation